Chordoid sarcoma (extraskeletal myxoid chondrosarcoma)

Evaluation of a series of 12 chordoid sarcomas suggests that there is a wider range of histological features in this entity then previously appreciated. Six of the lesions had a typical tumor cell organization and a mixture of cellular and myxoid stromal components, while the remaining cases were atypical because of a more solid growth pattern. Four of the 12 cases, that included both typically myxoid and more cellular examples, had small foci with hyalinized stroma segragating individual or small groups of tumor cells with and without lacunar spaces. Two atypical cases revealed more extensive and obvious chondrocytic differentiation in recurrent or metastatic lesions and in one of these, the histological pattern was that of mesenchymal chondrosarcoma. Ultrastructural examination of three cases revealed fine structural features of both the tumor cell population and extracellular matrix compatible with chondrocytic differentiation. Results of light and electron microscopy of this series of chordoid sarcoma add further support for categorizing this tumor with other malignant chondrocytic neoplasms. It is probable that chordoid sarcoma and extraskeletal myxoid chondrosarcoma represent the same entity and that this lesion has a close histogenetic relationship to mesenchymal chondrosarcoma.